Wang Cui-zhong, Hou Ying-yong, Shen Kun-tang, Wang Hong-shan, Qin Jing, Sun Yi-hong, Qin Xin-yu
Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
Zhonghua Wei Chang Wai Ke Za Zhi. 2011 Aug;14(8):599-602.
To investigate the clinicopathological and molecular genetic characteristics of gastrointestinal stromal tumor (GISTs) with significant cystic changes, and to assess their biological behavior.
Clinicopathological features of 7 patients with cystic GISTs treated at the Zhongshan Hospital of Fudan University from February 2005 to January 2010 were summarized retrospectively. The mutations status of c-kit and PDGFR-α were analyzed.
There were 2 males and 5 females aged from 46 to 76 years old. Primary site of GISTs included stomach(n=4), duodenum(n=1), and small intestinal(n=2). Tumor size ranged from 6 to 16 cm with obviously cystic changes. Tumor cells were found in the solid components under microscope, of which epithelioid cell type were found in 4 case and spindle cell type in 3 cases. The mitotic figures were no more than 3/50 HPF in all the patients. According to the NIH criteria, 4 were high-risk and 3 were low-risk. Based on morphological characteristics, 3 cases were as borderline tumor, 3 moderate-risk, and 1 moderate-risk. Gene mutation of exon 11 of c-kit were identified in 3 cases. During the follow up ranging from 9 to 80 months, all the 7 patients had cancer-free survival.
The biological behavior of cystic GIST is indolent with a low risk of malignancy and favorable prognosis.
探讨具有显著囊性变的胃肠道间质瘤（GISTs）的临床病理及分子遗传学特征，并评估其生物学行为。
回顾性总结2005年2月至2010年1月在复旦大学附属中山医院接受治疗的7例囊性GIST患者的临床病理特征。分析c-kit和PDGFR-α的突变状态。
患者年龄46～76岁，男2例，女5例。GISTs的原发部位包括胃（4例）、十二指肠（1例）和小肠（2例）。肿瘤大小6～16 cm，伴有明显囊性变。镜下见实性成分中有肿瘤细胞，其中上皮样细胞型4例，梭形细胞型3例。所有患者的核分裂象均不超过3/50 HPF。根据美国国立卫生研究院（NIH）标准，4例为高危，3例为低危。根据形态学特征，3例为交界性肿瘤，3例为中度危险，1例为中度危险。3例检测到c-kit基因第11外显子突变。随访9～80个月，7例患者均无瘤生存。
囊性GIST生物学行为惰性，恶性风险低，预后良好。
